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Solid Tumour Section
Review
Bone: Chondroma
Roberta Vanni
Dip. Scienze e Tecnologie Biomediche, Sezione di Biologia e Genetica, Universitá di Cagliari, Cittadella
Universitaria, 09142 Monserrato, Italy (RV)
Published in Atlas Database: May 2003
Online updated version: http://AtlasGeneticsOncology.org/Tumors/ChondromaID5147.html
DOI: 10.4267/2042/37994
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence.
© 2003 Atlas of Genetics and Cytogenetics in Oncology and Haematology
proximal and distal parts of the femur and the proximal
part of the humerus.
Identity
Note
Chondroma is an uncommon benign tumour which
characteristically forms mature cartilage. It is found
mostly in the small bones of the hand and/or feet,
although it can also occur in long, tubular bones,
primarily the humerus, femur and ribs. Occasion-ally,
focal areas of mixoid degeneration may result in a
mistaken diagnosis of chondrosarcoma.
Classification
Chondromas are classified according to their location:
- enchondroma: within the bone (within the medullary
cavity),
- periosteal chondroma: on the surface of the bone,
- soft tissue chondroma in the soft tissue.
Clinics and pathology
Disease
Enchondroma
Note
Enchondroma is usually a solitary benign lesion in
intramedullary bone. Usually asymptomatic, it is
incidentally discovered as a palpable bony nodule.
Rarely, causes soft tissue swelling and pain at the
lesion site. Pain can be a sign of pathologic fracture.
Both sex are equally affected, and any age group can be
involved. It is thought to develop from epiphyseal
cartilage rests that subsequently proliferate and slowly
enlarge. Approximately 50% of solitary enchondromas
are found in the hands, typically in the middle and
distal portions of the metacarpals and the proximal
portions of the phalanges, 10% in the feet, 20% in the
Atlas Genet Cytogenet Oncol Haematol. 2003; 7(3)
Fig: Enchondroma in the distal portion of the femur shaft.
(Courtesy of Dr Henry DeGroot at http://www.drdegroot.com).
On gross examination, the lesion is well circum-scribed
and has the pale bluish-gray appearance typical of
cartilage.
The nonerheditary syndrome of multiple enchondromas or enchondromatosis is known as Ollier's
disease. Enchondromatosis associated with soft tissue
hemangiomas is known as Maffucci's syndrome.
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Bone: Chondroma
Vanni R
Solitary lesion in the hand rarely undergoes
transformation. It has been suggested that Maffucci’s
syndrome is associated with a very high incidence of
malignancy, either in the skeleton or in visceral organs.
Pathology
Microscopically, enchondroma is hypocellular with few
double-nucleated cells without cytologic atypia, but
cellularity may vary. There is no permeation of
morrow. The matrix does not show any myxoid change.
Calcification and ossification are common. Histologic
appearance of enchon-droma may recall that of a grade1 chondro-sarcoma. The permeation through the cortex
into soft tissue must be identified before a diagnosis of
chondrosarcoma is made.
The chondromas in Ollier disease and Maffucci
syndrome may demonstrate a greater degree of
cellularity and cytologic atypia, and may be difficult to
distinguish from chondrosarcoma.
Disease
Periosteal chondroma
Note
a) Fig: Ankle periosteal chondroma; (courtesy of Dr Nick Ordall
http://www.xray2000.f9.co.uk/).
b) Fig: Chondroma of the right femur (courtesy of Dr Henry
DeGroot at http://www.drdegroot.com/).
Periosteal chondroma is a painful cartilaginous lesion
that arises from surface of cortex deep to the
periosteum, producing broad based cartilaginous mass
that may extend into soft tissues; often develops after
adolescence. It does not infiltrate the adjacent soft
tissue but may increase in size. It is similar in
appearance and location to periosteal osteosarcoma.
The potential for confusion with periosteal and
parosteal osteosarcoma mandates a thorough
investigation and biopsy of this lesion. The most
common location is adjacent to the metaphysis. The
cortex may be involved to a variable degree, but the
lesions do not involve the medullary space.
Fig: H&E 20x original magnification of an enchondroma: note
lobules of benign cartilage cells and hyaline matrix. (Courtesy of
Dr Henry DeGroot at http://www.drdegroot. com).
Treatment
No treatment is required for asymptomatic lesions. If
fracture occurs it is usually treated with curretage and
bone grafting.
Pathology
It persists as mass of mature cartilage. Low power
microscopy shows well circumscribed lobulated
hyaline masses. Cellularity can vary, from hypo- to
hyper-cellularity. The cartilage looks more active than
enchondroma and the lesion may be confused with
chondrosarcoma.
Evolution
A small percentage of enchondromas will undergo
malignant transformation, usually throught a slow
process, occurring over decades. It is more common in
long bones than short.
Treatment
Prognosis
Periosteal chondromas are treated with conservative
excision.
Prognosis for benign enchondroma is excellent.
Atlas Genet Cytogenet Oncol Haematol. 2003; 7(3)
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Bone: Chondroma
Vanni R
Prognosis
Treatment
Risk of recurrence after bloc marginal excision is less
than 10%.
Local surgery is the treatment of choice.
Genetics
Note
Cytogenetic studies of chondromas are scarse. A total
of 16 cases with abnormal karyotypes have been
reported: 6 enchondromas, 4 periosteal chondromas,
and 6 soft part chondromas. No consistent abnormality
has been detected, although chromosome or
chromosomal region 4, 5, 6, 7 and 12q13-15 seems to
be nonrandomly involved in changes.
References
Bridge JA, Persons DL, Neff JR, Bhatia P. Clonal karyotypic
aberrations in enchondromas. Cancer Detect Prev.
1992;16(4):215-9
Bridge JA, Bhatia PS, Anderson JR, Neff JR. Biologic and
clinical significance of cytogenetic and molecular cytogenetic
abnormalities in benign and malignant cartilaginous lesions.
Cancer Genet Cytogenet. 1993 Sep;69(2):79-90
Mandahl N, Willén H, Rydholm A, Heim S, Mitelman F.
Rearrangement of band q13 on both chromosomes 12 in a
periosteal chondroma. Genes Chromosomes Cancer. 1993
Feb;6(2):121-3
Sandberg AA Bridge JA. The Cytogenetics of bone and soft
tissue tumors. Austin: R.G. Landes Company; 1994.
Fig: Bone tumor images (courtesy of Dr Henry DeGroot at
http://www.drdegroot.com)
Dal Cin P, Qi H, Sciot R, Van den Berghe H. Involvement of
chromosomes 6 and 11 in a soft tissue chondroma. Cancer
Genet Cytogenet. 1997 Feb;93(2):177-8
Disease
Gunawan B, Weber M, Bergmann F, Wildberger J, Füzesi L.
Solitary enchondroma with clonal chromosomal abnormalities.
Cancer Genet Cytogenet. 1998 Jul 15;104(2):161-4
Soft-tissue chondroma
Note
Soft-tissue chondroma is a benign cartilage-forming
tumor, usually arising from tenosynovial sheaths or the
soft tissue adjacent to tendons in the hands and feet,
usually without any connection to the under-lying bone.
Predominantly sited in the fingers, it is usually solitary,
develops in adults, and may causes pain. It is composed
entirely of mature hyaline cartilage. Infrequently, the
tumor undergoes secondary changes and may exhibit
morphologic features that result in diagnostic difficulty.
Shadan FF, Mascarello JT, Newbury RO, Dennis T, Spallone
P, Stock AD. Supernumerary ring chromosomes derived from
the long arm of chromosome 12 as the primary cytogenetic
anomaly in a rare soft tissue chondroma. Cancer Genet
Cytogenet. 2000 Apr 15;118(2):144-7
McDermott AL, Dutt SN, Chavda SV, Morgan DW. Maffucci's
syndrome: clinical and radiological features of a rare condition.
J Laryngol Otol. 2001 Oct;115(10):845-7
Tallini G, Dorfman H, Brys P, Dal Cin P, De Wever I, Fletcher
CD, Jonson K, Mandahl N, Mertens F, Mitelman F, Rosai J,
Rydholm A, Samson I, Sciot R, Van den Berghe H, Vanni R,
Willén H. Correlation between clinicopathological features and
karyotype in 100 cartilaginous and chordoid tumours. A report
from the Chromosomes and Morphology (CHAMP)
Collaborative Study Group. J Pathol. 2002 Feb;196(2):194-203
Pathology
Microscopically,
soft-tissue
chondromas
vary
considerably in appearence. Most consist of hyaline
cartilage arranged in lobular pattern, and may show
focal fibrosis, ossification, or myxoid change. Diffuse
calcification may occur, completely obscuring the
cartilagineous nature of the lesion. In some variants, the
cartilage matrix becomes extensively mineralized, often
associated with necrosis of chondrocytes, causing the
tumor to resemble tumoral calcinosis. Hyaline cartilage
may also undergo enchondral ossification, mimicking
an osteogenic neoplasm or a reactive lesion. Myxoid
degeneration may create confusion with extra-skeletal
myxoid chondrosarcoma.
Atlas Genet Cytogenet Oncol Haematol. 2003; 7(3)
Buddingh EP, Naumann S, Nelson M, Neffa JR, Birch N,
Bridge JA. Cytogenetic findings in benign cartilaginous
neoplasms. Cancer Genet Cytogenet. 2003 Mar;141(2):164-8
Sandberg AA, Bridge JA. Updates on the cytogenetics and
molecular genetics of bone and soft tissue tumors:
chondrosarcoma and other cartilaginous neoplasms. Cancer
Genet Cytogenet. 2003 May;143(1):1-31
This article should be referenced as such:
Vanni R. Bone: Chondroma. Atlas Genet Cytogenet Oncol
Haematol. 2003; 7(3):191-193.
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